Unilateral Primary Angiitis of the Central Nervous System: A Rare and Under-Recognized Entity.
WHY IT MATTERS
If you've been diagnosed with a one-sided brain lesion or inflammation that doctors can't fully explain, this research helps clarify that U-PACNS should be considered in your differential diagnosis, potentially changing your treatment approach.
Doctors have identified a rare form of brain inflammation called unilateral primary angiitis of the central nervous system (U-PACNS) that affects only one side of the brain instead of both sides. This condition is often missed or confused with other diseases like brain tumors or multiple sclerosis because it looks similar on imaging tests. The article explains how doctors can better recognize and diagnose this condition by understanding its unique features.
Unilateral Primary Angiitis of the Central Nervous System: A Rare and Under-Recognized Entity. Abstract: Primary angiitis of the central nervous system (PACNS) is an uncommon inflammatory vasculitis restricted to the brain, spinal cord, and/or the leptomeninges. While typically bilateral, a rare subset presents with strictly unilateral involvement. This variant remains underrecognized and is often misdiagnosed as glioma, demyelination, or chronic encephalitis due to overlapping clinical and imaging features. Unilateral PACNS (U-PACNS) may manifest as tumor-like solitary or multifocal lesions, infarcts, or hemorrhages. Imaging, including vessel wall imaging, perfusion, and spectroscopy may demonstrate underlying abnormalities but is often nonspecific. Histopathologically, most cases show small- to medium-vessel lymphocytic vasculitis, in contrast to granulomatous inflammation, which is more co Authors: Rai et al. Journal: AJNR. American journal of neuroradiology MeSH: Humans, Vasculitis, Central Nervous System, Diagnosis, Differential, Male, Female, Middle Aged, Rare Diseases
ASK YOUR DOCTOR
If you have unilateral (one-sided) brain lesions or inflammation that remain undiagnosed, ask your neurologist whether they've considered unilateral PACNS and whether vessel wall imaging or a brain biopsy might help confirm the diagnosis.